Stander Use in Spinal Muscular Atrophy: Results From a Large Natural History Database

Pediatr Phys Ther. 2020 Jul;32(3):235-241. doi: 10.1097/PEP.0000000000000713.

Abstract

Purpose: The purpose of this study was to describe stander use in a natural history cohort of drug therapy-naïve children with spinal muscular atrophy (SMA) who are not walking and identify factors associated with consistent stander use.

Methods: Data from 397 children with SMA types 1 and 2 characterized the prevalence and frequency of stander use. Predictors of consistent stander use explored were SMA type, survival motor neuron 2 gene (SMN2) copy number, respiratory support, and motor performance.

Results: Prevalence of consistent stander use was 13% in type 1 and 68% in type 2. SMA type, SMN2 copy number, respiratory support, and head rotation control each predicted consistent stander use.

Conclusions: Findings characterize stander use in children with SMA who are not walking, address important safety considerations, identify factors that may inform physical therapists' clinical decision-making related to standing program prescription, and provide guidance for future prospective studies.

Publication types

  • Research Support, N.I.H., Extramural
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Child
  • Child, Preschool
  • Cohort Studies
  • Female
  • Gene Dosage
  • Humans
  • Infant
  • Infant, Newborn
  • Male
  • Motor Skills Disorders / rehabilitation*
  • Practice Guidelines as Topic*
  • Prospective Studies
  • Rehabilitation / standards*
  • Rehabilitation / statistics & numerical data*
  • Retrospective Studies
  • Spinal Muscular Atrophies of Childhood / genetics*
  • Spinal Muscular Atrophies of Childhood / rehabilitation*
  • Standing Position*